Dysplasia Epiphysealis Capitis Femoris (capital dysplasia) is a clinical — radiologic syndrome which resembles Perthes' disease but has important differences, especially in its course and prognosis. Its radiographs show no collapse or gross fragmentation of the femur. The structure of the head is granular but the final result is normal structure and a normal shape of the head. Progressiv improvement of the radiologic appearance of the epiphysis characterizes capital dysplasia. We found two cases among 33 patients with the diagnosis of Perthes' disease. These showed progressiv improvement of the radiologic appearance and developed a normal or nearly spherical femoral head but the left hip of case I that developed Perthes' disease.
Hereditary hyperphosphatasia is a rare familial disease characterised by skeletal abnormalities, that is, cortical thickening and bowing of long bones and thickening of the skull bone, and high serum level of alkaline phosphatase. The disease usually appears in childhood. Female, 44, had onset of leg deformities at age 12. Deformity of facial bone had progressed during third decade. Hearing was reduced at age 40. Roentgenologically, the bone of the cranial vault was thickened and sclerotic. Cortical thickening and lateral bowing of the long bone was characteristic. The alkaline phosphatase level was high.
Gagroylism, congenital disorder of mucopolysaccharide metabolism, was first described by Hunter in 1917. Its main clinical features are as follows; 1) mental retardation, 2) dwarfism, 3) Gargoyle appearance, 4) hepatomegaly and splenomegaly and 5) corneal opacity. Recently, we have observed a ten-years-old boy who suffered from protuberant abdomen and retardation of growth. Clinically, a limitation in joints movement and an umbilical herniation as well as the above mentioned features except corneal opacity, were existed. On X-ray examination, an enlarged Turkish saddle, oar-shaped ribs, cocave lenz-shaped vertebral bodies, coxa valga and hypoplastic carpalia were observed. We performed tetracycline bone labelling in this case. The number of tetracycline labelled osteons per mm2 of cortical cross section (symbol; Af) was 1.0 in the eleventh rib of this case. It was less than the Af in 10 age normal subjects.
Thirty-two patients with the developmental bone disease were registered in the department of orthopedic surgery, Tottori university school of medicine, for the past three years. The authors made a description of the clinical features and radiologic findings of the following patients. 1) Chondrodysplasia punctata (an one-year-old young girl) 2) Chondroetodermal dysplasia (a two-month-old baby) 3) Hypothyroidism with circulatory disturbance in the lower extremities (a twenty-six-old male) Then, the origin and inheritance of these diseases were discussed.
A six-year-old boy with Lesch Nyhan syndrome was reported. The patient, who had been previously diagnosed of cerebral palsy, showed hyperuricemia (11.6mg/dl), self mutilation, mental retardation, dystonic movement and hypoxanthine-guanine phosphoribosyltran-ceferase (HGPRT) deficiency. Other two cases with hyperuricemia, asymptomatic father and a brother with signs of Lesch Nyhan syndrme, were found in the same family and consanguinity was proved.
We encountered two patients who showed the characteristic features of the Marinesco-Sjögren syndrome. They showed cerebellar ataxia, congenital cataract and retarded physical and mental maturation. Pes planovalgus and brachydactylia of 4th toe were seen in both cases. In addition, elongation of the index finger was found in one case. The finding of pneumoventriculography showed the atrophy of the cerebellum. Their parents were consanguineous.
A 24-years-old male with hemophilia-A developed a large hemophilic pseudotumor of the right thigh, which was treated by subtrochanteric amputation of the femur. The preoperative level of antihemophilic globulin (A. H. F.) was 0.5%, and clotting time was prolonged over 35 minutes. The dose of 30u/kg of A. H. F. was administered preoperatively and 3 vials were added during operation. Postoperatively the dose of 30u/kg/day of A. H. F. was given by the method of divided drip infusion three times a day for a week, following by gradual decrease of the doses. The wound was partially opend twice and healed 10 weeks after the operation.
Interesting clinical pictures of two cases with intra-spinal anomalies inciuding diastematomyelia were presented. Case 1, 7 years old girl, had complained of the foot deformity and anal atresia. From the radiographical and the surgical findings, she proved to have partial diastematomyelia, lumbosacral vertebral anomalies and sacral agenesis. The conus medullaris was located at the sacral level. Case 2, 1 year old girl, complaining congenital scoliosis. The presice radiographic investigation suggested diastematomyela at L3-L5. The surgical procedure demonstrated a bony septum dividing the dural tube and a lipoma in the right 51 root. The necessity to prevent the development of the neurological disorders by early detection was emphasised.
From June 1969 through September 1974, seven patients with tumor of the cauda equina were operated on in our hospital. The most common symptoms were pains in the lumbar region and legs at the onset and the five cases out of seven were first treated as protruded intervertebral discs until referred to our clinic. Pathological findings were as follows: neurilemmoma in four cases, meningioma, malignant schwannoma and lipoma in one case respectively. It is convinced that early accurate diagnosis is important for preventing of permanent residual paralysis.
Partial or complete acromionectomy has been advocated for the operative treatment for chronic bursitis and partial or complete tears of the rotator cuff. Neer II emphasized that Impingement of the rotator cuff as one of the cases of chronic disability of the shoulder lied the anterior part of the undersurface of acromion. For the past one-half years, anterior acromioplasyt has been done in 6 cases of the shoulder lesions in our department. This paper discribed also in reference to anatomical findings of inspection of 60 dissected scapulae with special attension to the anterior part of acromion.
The purpose of this article is to review briefly current concepts of pathophysiology of this syndrome, and to describe the status of autonomic nerve systems. Six cases in this series were analysed with regard to their thermography, histamine flare test, angiography, sudomotor test and the tissue clearance by 131NaI.
24 patients he ving congenital deformities in finger and toe were treated in our hospital in the last thr ee and a half years, which corresponded to 0.56% of all outpatients during the period. Among 24 cases, operations were performed on 14 patients (60%). Having examined the results of the operations, we had no trouble in cases of congenital deformities in toes, but could not gain good result from the case of ossal syndactyly. No. of case Syndactyly 12 Polydactyly 8 Brachydactyly 5 Camptodactyly 4 Clinodactyly 4 Cleft hand (cleft foot) 3 Floating thumb 1 Peripheral Hypoplasia 1
We have experienced 4 cases of Dupuytren contracture during past one year. Our incidence of sex (3 male, 1 female), age (from 47 to 80, average 63.3) and finger involvement (3rd, 4th, 5th finger) were similar to many other reports. An operative procedure was performed on two cases (case 3, case 4). Case 3: Age 47, male, a office worker, became aware of subcutaneous indurations in the left palm about 10 years ago and gradually became suffering from contracture of the 3rd, 4th and 5th finger. He also noted indurations in the right palm and both plantar redions about 2 years ago. On February 4th 1974, a partial aponeurectomy was done to the left palm and to the right palm on April 10th. No recurrence and extension have appeaared even 8 or 10 months passed since the opration was performed. Case 4: Age 54, female, a manual worker, was caught in a washing machine and a plaster cast was applied for 2 weeks under the diagnosis of the styloid process fracture of the left ulna. She became aware of indurations in the left palm about 4 weeks after injuried and gradually became suffering from contracture of 3rd, 4th and 5th finger. Recently she noted indurations in the right palm and both plantar regions. On May 24th 1974, a partial aponeurectomy was done to the left palm, but finger contractures have recurred 6 months after operation because of the lack of post-operative physical treatment. This case may be so-called “post-traumatic Dupuytren contracture.”
Thumb-in-palm deformity in which thumb is adducted and flexed in palm is caused by many disorders. This deformity not only prevents the thumb from functioning in pinch but also prevents the hand functioning in grasp and release. And so correction of the thumb-in-palm deformity is very important for function of upper extremity. We have treated this deformities which are mainly associated with cerebral palsied hand by conservative and surgical treatment at Adachi Gakuen Hospital. Ten patients, 14 hands, in which 7cases were C. P., 2 cases were arthrogryposis multiplex congenita and 1 case was congenital clasped thumb were selected for surgical treatment. The results of surgical treatment were relatively good in grasp release function, pinch, activity of daily living and appearance in many cases.
We present the experiences of 30 operations in 12 patients in the use of lateral rotation flap. We find some merits during the performance of lateral rotation flap. (1) The joint can be covered by the flap. (2) Through the flap, arthroplasty and tendon suture can be performed later. (3) The elasticity of the joint can be gained. (4) The flap is also in good result in gross appearance. For the excellent result of the lateral rotation flap, we apply it not only in the dorsal digital surface but also to reconstruct the joint of knee and axillary area.
We tried the reattachment of the amputated finger with handling microscope in 14 cases of complete and incomplete ones. Results were satisfactory in 11 cases and failure in 3 cases. We tried Arterio-Venus Shunt with the vein graft, because of the failure in vein suture at D. I. P. Joint of the amputated finger in spite of success in the anastomosis of digital artery. We used the anticoagulant for two weeks but had no secondary reaction. Angio graphy of 20 normal hands were performed. Proper volar digital artery discussed in diameter.
One case, five years old boy, suffering from deep and wide burn involving in iliac bone and vertebrae was repored. At first a necrotic tissue was removed and then the wound was covered with human amnion membrane two times. In this case, the Sulfamylon cream was effective for depressing infction of pseudomonas aeruginosa. A brace was made for the training of ambulation and maintaining the range of motion of hip joints. A special strecher type' bed was made by the Devillon-mesh-cloth. He discharged on 146th hospital day, and he had no restriction for activities of daily living.
We experienced of children with cerebral palsy at our hospital and reviewed the next items. 1) Incidence 2) Relationship between patella alta and range of the knee motion. 3) Relationship between patella alta and age. 4) Relationship between patella alta and genu recurvatum of the knee. Patella alta observed in 24 cases (56%) of spastic children and 6 cases (30%) of athetoid children. In spastic children, patella alta increased with age. The range of active knee motion decreased first, then those of passive motion decreased later with age. The number of patients with genu-recurvatum who did not have patella alta was observed more frequently in athetoid than in spastic children.
Rupture of the Achilles tendon is a common injury, but subcutaneous partial rupture has been regarded as uncommon. Some has denied its existence. Recently we diagnosed three male patients of the fourth decade as subcutaneous partial rupture and treated by operation. In all cases the site of the rupture located at the medial portion of the tendon, one about 3cm and the others about 10cm above the insertion.
Pes anserinus syndrome was first described by B. Calandriello in 1973. This syndrome is characterized by pain in the part of the pes anserinus, which is often elicited by flexion of the knee against resistance and by internal rotation of the leg when standing. Recently, we experienced three cases of this syndrome out of 240 outpatients with complaints of pain in the knee. All cases, which were 64-year-old, 55-year-old and 44-year-old females, were treated by local steroid infiltration with rapid resolution.
Peroneal compartment syndrome is uncommon, and only 5 cases have been recorded in the world's literatures. A 19 year-old man who suffered from this syndrome after the trainig of Rugby football is reported. He came to our clinic at fifth day of the onset, complaining severe pain on his left leg. At emergency operation, rupture of peroneus longus muscle and much bloody coagulation were observed in the distended compartment, and both peroneal muscles appeared to be severely ischemic and necrotic, so, total excision of the muscles was carried. In spite of the muscular defects, functional loss of his ankle joint is not remarkable. He have recovered so good as to play football since seven months after the surgery.
A case is a 74 years-old male who has a history of psoriasis at the both lower legs and feet two years ago. He was affected with arthritis of the knee, followed by erythroderma. Radiographical examinations showed marked destructive joint change of the right knee and narrowing of DIP joint of fingers and toes. Rheumatoid factor in the serum was negative. Remission of joint manifestation tended to occur synchronously with that of erythroderma. It seems that arthritis of right knee was associated with erythroderma by the clinical features and examinations.
We experienced a rare case of the tuberculous arthritis of the right knee joint with a large popliteal cyst. The patient was 67 years old male suffering from right gonalgia since 7 years. Two years ago he became aware of the cyst at the popliteal region. With cystgraphy the cyst consisted of two parts was recognized. These were communicating with each other at th point of tibio-fibular joint and perforated into the joint. The cyst and the joint filled with tuberculous effusion. We considered that the origin of these cysts was the lateral gastrocnemius bursa and popliteus bursa. Histopathological study showed tuberculous-change in these cyst wall.